LMNA blocking peptide

LMNA Antibody (Center) Blocking Peptide

• Gene Names: LMNA; FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B
• Synonyms: LMNA; LMNA Antibody (Center) Blocking Peptide; Prelamin-A/C; Lamin-A/C; 70 kDa lamin; Renal carcinoma antigen NY-REN-32; LMN1; LMNA blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 664

• Cellular Location: Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Note: Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin- A/C, which can then be inserted into the nuclear lamina. EMD is required for proper localization of non-farnesylated prelamin-A/C
• Tissue Location: In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for LMNA blocking peptide

Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone.

NCBI and Uniprot Product Information

• NCBI GI #: 125962
• NCBI GeneID: 4000
• UniProt Accession #: P02545; P02546; Q5I6Y4; Q5I6Y6; Q5TCJ2; Q5TCJ3; Q6UYC3; Q969I8; B4DI32; D3DVB0; D6RAQ3; E7EUI9
• Molecular Weight: 69,249 Da
• NCBI Official Full Name: Prelamin-A/C
• NCBI Official Synonym Full Names: lamin A/C
• NCBI Official Symbol: LMNA
• NCBI Official Synonym Symbols: FPL; IDC; LFP; CDDC; EMD2; FPLD; HGPS; LDP1; LMN1; LMNC; PRO1; CDCD1; CMD1A; FPLD2; LMNL1; CMT2B1; LGMD1B
• NCBI Protein Information: lamin
• UniProt Protein Name: Prelamin-A/C
• Protein Family: Lamin
• UniProt Gene Name: LMNA
• UniProt Synonym Gene Names: LMN1
• UniProt Entry Name: LMNA_HUMAN

NCBI Description

The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Alternative splicing results in multiple transcript variants. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome. [provided by RefSeq, Apr 2012]

Uniprot Description

lamin A/C: nuclear lamins are intermediate filament proteins that constitute the lattice-like matrix at the inner face of the nuclear membrane that underlies the nuclear envelop. The lamins, highly conserved throughout evolution, are encoded by three genes in the human: LMNA, LMNB1, and LMNB2. The A-type lamins (lamin A/C) are developmentally regulated and are generally expressed in differentiated cells. The anchoring of chromatin to the nuclear lamina is involved in the control of gene expression and in DNA replication and repair. During mitosis, the nuclear lamina is reversibly disassembled as the lamin proteins are phosphorylated. Cleaved by caspase-6 during apoptosis into a 40-45 kDa and a28 kDa fragment.

Protein type: Cytoskeletal

Chromosomal Location of Human Ortholog: 1q22

Cellular Component: cytoplasm; cytosol; nuclear envelope; nuclear membrane; nucleoplasm; nucleus

Molecular Function: protein binding

Biological Process: establishment and/or maintenance of microtubule cytoskeleton polarity; mitotic nuclear envelope disassembly; mitotic nuclear envelope reassembly; regulation of cell migration

Disease: Cardiomyopathy, Dilated, 1a; Cardiomyopathy, Dilated, With Hypergonadotropic Hypogonadism; Charcot-marie-tooth Disease, Axonal, Type 2b1; Emery-dreifuss Muscular Dystrophy 2, Autosomal Dominant; Emery-dreifuss Muscular Dystrophy 3, Autosomal Recessive; Heart-hand Syndrome, Slovenian Type; Hutchinson-gilford Progeria Syndrome; Lipodystrophy, Familial Partial, Type 2; Mandibuloacral Dysplasia With Type A Lipodystrophy; Muscular Dystrophy, Congenital, Lmna-related; Muscular Dystrophy, Limb-girdle, Type 1b; Restrictive Dermopathy, Lethal